A prospective study to assess the impact of a novel CFTR therapy combination on body composition in patients with cystic fibrosis with F508del mutation

Knott-Torcal, Carolina; Sebastián-Valles, Fernando; Girón Moreno, Rosa María; Martín- Adán, José Carlos; Jiménez-Díaz, Jessica; Marazuela, Mónica; Sánchez de la Blanca, Nuria; Fernández-Contreras, Raúl; Arranz-Martín, Alfonso

doi:10.1016/j.clnu.2023.10.015

Cited by 3 publications

(2 citation statements)

References 19 publications

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“…85 However, data remain limited and, in particular, greatly limited in respect to the newer triple combination ETI CFTRms. Although early data are emerging on this in both adult and paediatric groups, 86,87 it remains too early to draw firm conclusions. Results are increasingly appearing in abstract form, and findings from ongoing research in this area are expected soon.…”

Section: Changing Nutritional Profilefrom Undernutrition To Overnutri...mentioning

confidence: 99%

Evolution of nutritional management in children with cystic fibrosis – a narrative review

Smith,

Lowdon,

Noordhoek

et al. 2024

J Human Nutrition Diet

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Nutrition has played a central role in the management and outcomes of people with cystic fibrosis (pwCF) since the 1970s. Advances in therapies and practices in recent decades have led to a significant change in the patient landscape with dramatic improvements in life expectancy, as well as quality of life, bringing with it new issues. Historically, cystic fibrosis was a condition associated with childhood and malnutrition; however, changes in patient demographics, nutritional assessment and fundamental nutritional management have evolved, and it has become an increasingly prevalent adult disease with new nutritional challenges, including obesity. This paper aims to describe these changes and the impact and challenges they bring for those working in this field. Nutritional professionals will need to evolve, adapt and remain agile to the wider range of situations and support required for a new generation of pwCF. Specialised nutrition support will continue to be required, and it will be additionally important to improve and optimise quality of life and long‐term health.

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Section: Changing Nutritional Profilefrom Undernutrition To Overnutri...mentioning

confidence: 99%

Evolution of nutritional management in children with cystic fibrosis – a narrative review

Smith,

Lowdon,

Noordhoek

et al. 2024

J Human Nutrition Diet

View full text Add to dashboard Cite

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“…Также у пациентов с МВ (n = 36) на фоне приема тройной комбинации элексакафтор / тезакафтор / ивака фтор через 6 мес. лечения наблюдалось значительное увеличение ИМТ и ЖМТ [13,14]. В исследовании L.R.…”

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Nutritional support for adult сystic fibrosis patients receiving targeted treatment

Krylova,

Amelina,

Krasovsky

et al. 2024

Pulʹmonologiâ (Mosk.)

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Cystic fibrosis (CF) is characterized by the development of a severe nutritional deficiency. A low BMI directly correlates with low lung function and requires active nutritional support. Pathogenetic (targeted) therapy aimed at restoring the chlorine channel function also leads to weight gain. The effects of CFTR modulators on extrapulmonary pathology in adult CF patients in Russia have been described very little.Aim. To evaluate the sequential impact of two targeted drugs – the potentiator ivacaftor and the triple combination of CF transmembrane regulator modulators elexacaftor/tezacaftor/ivacaftor – on the nutritional status of an adult patient with cystic fibrosis receiving nutritional support.Conclusion. Therapy with CFTR modulator in combination with sipping nutritional support promotes significant weight gain in adult CF patients. The triple combination of elexacaftor/tezacaftor/ivacaftor has a more active effect on nutritional status than ivacaftor alone. The targeted therapy requires supervision by a nutritionist.

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Impact of CFTR modulator therapy on body composition as assessed by thoracic computed tomography: A follow-up study

Navas-Moreno,

Sebastian-Valles,

Rodríguez-Laval

et al. 2024

Nutrition

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A prospective study to assess the impact of a novel CFTR therapy combination on body composition in patients with cystic fibrosis with F508del mutation

Cited by 3 publications

References 19 publications

Evolution of nutritional management in children with cystic fibrosis – a narrative review

Evolution of nutritional management in children with cystic fibrosis – a narrative review

Nutritional support for adult сystic fibrosis patients receiving targeted treatment

Impact of CFTR modulator therapy on body composition as assessed by thoracic computed tomography: A follow-up study

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